| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | ATPAF1 |
| Uniprot No | Q5TC12 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-328aa |
| 氨基酸序列 | MAAVVVAAAGGAGPAVLQVAGLYRGLCAVRSRALGLGLVSPAQLRVFPVRPGSGRPEGGADGSGVGAEAELQANPFYDRYRDKIQLLRRSDPAAFESRLEKRSEFRKQPVGHSRQGDFIKCVEQKTDALGKQSVNRGFTKDKTLSSIFNIEMVKEKTAEEIKQIWQQYFAAKDTVYAVIPAEKFDLIWNRAQSCPTFLCALPRREGYEFFVGQWTGTELHFTALINIQTRGEAAASQLILYHYPELKEEKGIVLMTAEMDSTFLNVAEAQCIANQVQLFYATDRKETYGLVETFNLRPNEFKYMSVIAELEQSGLGAELKCAQNQNKT |
| 分子量 | 62.8 kDa |
| 蛋白标签 | GST-tag at N-terminal |
| 缓冲液 | 冻干粉 |
| 稳定性 & 储存条件 | Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt. Reconstituted protein solution can be stored at 2-8°C for 2-7 days. Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months. |
| 复溶 | Always centrifuge tubes before opening.Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100μg/ml. Dissolve the lyophilized protein in distilled water. Please aliquot the reconstituted solution to minimize freeze-thaw cycles. |
以下是关于ATPAF1(ATP synthase mitochondrial F1 complex assembly factor 1)的3篇代表性文献示例(文献为假设，用于说明格式)：
1. **文献名称**: "ATPAF1 is essential for the assembly of mitochondrial F1Fo-ATP synthase by interacting with the α and β subunits."
**作者**: Wang et al.
**摘要**: 研究揭示了ATPAF1通过与ATP合成酶的α和β亚基直接相互作用，调控线粒体F1复合物的组装过程，其缺失会导致ATP合成障碍及线粒体功能障碍。
2. **文献名称**: "ATPAF1 mutations cause neurodegeneration by impairing mitochondrial energy production."
**作者**: Smith et al.
**摘要**: 报道了ATPAF1基因突变与神经退行性疾病的相关性，发现突变会破坏ATP合成酶的组装，导致神经元能量代谢缺陷和细胞凋亡。
3. **文献名称**: "Functional analysis of ATPAF1 in cellular oxidative phosphorylation and tumor growth."
**作者**: García et al.
**摘要**: 通过体外实验证明，ATPAF1敲低抑制肿瘤细胞线粒体氧化磷酸化能力，影响ATP生成，并提出其在癌症代谢重编程中的潜在治疗靶点作用。
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**备注**：实际文献需通过PubMed(https://pubmed.ncbi.nlm.nih.gov)或Web of Science检索关键词“ATPAF1”或“mitochondrial ATP synthase assembly”，筛选与疾病、分子机制或结构相关的研究。
ATP synthase mitochondrial F1 complex assembly factor 1 (ATPAF1) is a nuclear-encoded protein essential for the assembly and functional integrity of the mitochondrial ATP synthase complex. This enzyme catalyzes the final step of oxidative phosphorylation, producing ATP via proton gradient-driven rotation of its F1Fo structure. ATPAF1 specifically facilitates the proper assembly of the F1 catalytic domain, a hexameric α3β3 subunit complex responsible for ATP synthesis. Acting as a molecular chaperone, ATPAF1 stabilizes nascent β-subunits, ensures their correct folding, and mediates their coordinated integration with α-subunits during F1 complex formation.
Mutations or deficiencies in ATPAF1 disrupt ATP synthase biogenesis, leading to impaired mitochondrial energy production. Human ATPAF1 is encoded by a gene located on chromosome 1p33. with pathogenic variants associated with early-onset mitochondrial encephalopathies, cardiomyopathy, and Leigh syndrome. Cellular studies show ATPAF1 knockdown causes defective F1 assembly, reduced ATP synthase activity, and fragmented mitochondrial networks. Its expression is tightly regulated in energy-demanding tissues, particularly brain, heart, and skeletal muscle. Recent research highlights ATPAF1's potential role beyond assembly, including regulation of mitochondrial protein translation and stress responses. As a diagnostic biomarker and therapeutic target, ATPAF1 continues to be investigated in mitochondrial disorders and neurodegenerative diseases linked to bioenergetic failure. (Word count: 214)
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